Case Report: Intracranial growing teratoma syndrome in two children with suprasellar mixed germ cell tumors: implications for the timing of surgery.
This two-patient case report describes pediatric suprasellar mixed germ cell tumors that enlarged with clinical deterioration despite falling tumor markers, with resection demonstrating mature teratoma consistent with intracranial growing teratoma syndrome and both patients subsequently remaining in remission but developing permanent hypothalamic-pituitary dysfunction.
Open original publication →What the AI sees
This two-patient case report describes pediatric suprasellar mixed germ cell tumors that enlarged with clinical deterioration despite falling tumor markers, with resection demonstrating mature teratoma consistent with intracranial growing teratoma syndrome and both patients subsequently remaining in remission but developing permanent hypothalamic-pituitary dysfunction.
Research significance
The cases provide evidence that symptomatic or radiographic progression during biochemical response can represent resectable iGTS; they support, but do not establish, the hypothesis that earlier surgical recognition and intervention in selected patients could relieve mass effect and reduce irreversible morbidity.
Source abstract
BACKGROUND: Intracranial growing teratoma syndrome (iGTS) is characterized by paradoxical tumor enlargement despite normalization of tumor markers during treatment of germ cell tumors. Its recognition is critical, particularly in the suprasellar region where mass effect can lead to significant morbidity. CASE PRESENTATION: We report two pediatric patients with suprasellar mixed germ cell tumors treated with alternating etoposide-cisplatin and ifosfamide-etoposide chemotherapy. In both cases, serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (β-HCG) levels declined markedly during treatment; however, clinical deterioration and radiologic tumor enlargement occurred. Surgical resection revealed mature teratoma in both cases, confirming classic iGTS after histopathologic review. Gross total resection was achieved in both cases, followed by completion of chemotherapy and craniospinal irradiation. At follow-up, both patients remained in remission but developed permanent hypothalamic-pituitary dysfunction. RESULTS: These cases illustrate a clear dissociation between biochemical response and local mass enlargement, consistent with classic iGTS in both patients. CONCLUSION: Declining tumor markers should not be interpreted as reassurance in the presence of worsening symptoms or tumor enlargement. Early surgical intervention should be considered in selected patients to relieve mass effect and potentially reduce long-term morbidity.